Alan Davidson

The management of children with Kaposi sarcoma in resource limited settings

Kaposi sarcoma (KS) is common where HIV infection is endemic. Antiretroviral therapy (ART) has reduced the incidence in well‐resourced settings but in some parts of the world access to ART is delayed. These recommendations are for use where only minimal requirements for treatment are available. Consensus was sought for the management of childhood HIV‐associated KS in this setting. There are no randomised controlled studies of chemotherapy for KS in children and these recommendations have drawn on consensus of a group of experts and published reports from studies in adults. Pediatr Blood Cancer 2013; 60: 538–542.

Malignancies in South African children with HIV.

Objectives: In 2008 the South African Children’s Cancer Study Group decided to review the epidemiology, management, and chemotherapy response of HIV-positive children with malignancy. Methods: This is a retrospective analysis of data collected from the records of HIV-positive children diagnosed with malignancy at 7 university-based pediatric oncology units serving 8 of the 9 provinces in South Africa. Results: Two hundred eighty-eight HIV-positive children were diagnosed with 289 malignancies between 1995 and 2009. Age at diagnosis ranged from 17 days to 18.64 years; median 5.79 years. Of the 220 HIV-associated malignancies, there were 97 Kaposi sarcomas, 61 Burkitt lymphomas, 47 other B-cell lymphomas including 2 primary central nervous system lymphomas, 12 Hodgkin lymphomas, and 3 leiomyosarcomas. Sixty-nine patients presented with non–AIDS-defining malignancies. More than 80% presented with advanced disease. Most patients (76.7%) were naive to antiretroviral therapy; 22.2% did not have access because it only became available in public hospitals in 2004. One hundred ninety-seven children were treated with curative intent; 91 received palliative care due to advanced malignancy and/or advanced HIV disease. Nearly one third had coexisting pathology, mostly tuberculosis. Overall survival for the whole group was 33.7%, but was 57.8% for those treated with antiretroviral therapy and chemotherapy. Conclusions: The study shows more Kaposi sarcoma and fewer primary central nervous system lymphomas among HIV-positive children than that is reported in the developed world, but confirms a higher incidence of non-Burkitt B-cell lymphoma than in HIV-negative children. The high number of non–AIDS-defining malignancies underscores the prevalence of HIV-AIDS in South Africa. The overall survival should improve with universal access to antiretrovirals and earlier diagnosis.

Kaposi sarcoma in children with HIV: a clinical series from Red Cross Children's Hospital

AIMnThe study aimed to assess the clinical presentation, diagnosis, and treatment of Kaposi sarcoma in our HIV-positive paediatric population; analyse the increase in Kaposi sarcoma with the HIV epidemic; and look at unique surgical presentations and their implications for gastrointestinal surveillance. The incidence of Kaposi sarcoma, a herpesvirus 8-associated neoplasm of endothelial cells, has increased with the onset of the HIV epidemic. Surgical interest was prompted by a case of Kaposi sarcoma presenting with intussusception.nnnMETHODSnAll HIV-positive children with Kaposi sarcoma from 2003 to 2007 were included in the study. A retrospective analysis of clinical data was performed.nnnMAIN RESULTSnThere were 9 children who ranged in age from 1 to 10 years (median, 7.1 years). Clinical presentations included oropalatal and laryngeal disease (5), pleural effusion (2), inguinoscrotal and skin disease (4), lymph node involvement (3), and gastrointestinal disease with haemorrhage (2), including one as a result of intussusception. Most patients presented with more than one clinical area involved. CD4 counts ranged from 14 to 2105. Two patients developed Kaposi sarcoma on antiretroviral treatment (ART); the remaining patients were started on antiretroviral treatment at presentation. Two patients died from overwhelming disseminated disease and one patient was lost to follow-up. Chemotherapy was required to achieve remission in the remaining 6. Before the HIV epidemic, this hospital treated one patient with Kaposi sarcoma every 4 years; the incidence has now increased to 2 patients per year.nnnCONCLUSIONnKaposi sarcoma is an increasingly important paediatric cancer in the era of the HIV epidemic. The clinical presentation in children is commonly oropalatal and inguinoscrotal disease. Gastrointestinal involvement was highlighted by a presentation with intussusception. The surgeons role in diagnosis and management includes clinical recognition, biopsy, and definitive treatment.

Childhood cancer in Africa

The majority of children with cancer live in low‐ and middle‐income countries (LMICs) with little or no access to cancer treatment. The purpose of the paper is to describe the current status of childhood cancer treatment in Africa, as documented in publications, dedicated websites and information collected through surveys. Successful twinning programmes, like those in Malawi and Cameroon, as well as the collaborative clinical trial approach of the Franco‐African Childhood Cancer Group (GFAOP), provide good models for childhood cancer treatment. The overview will hopefully influence health‐care policies to facilitate access to cancer care for all children in Africa. Pediatr Blood Cancer 2014;61:587–592.

Wilms tumour experience in a South African centre.

In Africa, Wilms tumour frequently presents with advanced disease. This study reports our results over 25 years using the National Wilms Tumour Study Group (NWTSG) approach of primary surgery followed by chemotherapy.

SIOP PODC adapted treatment recommendations for standard-risk medulloblastoma in low and middle income settings

Effective treatment of children with medulloblastoma requires a functioning multi‐disciplinary team with adequate neurosurgical, neuroradiological, pathological, radiotherapy and chemotherapy facilities and personnel. In addition the treating centre should have the capacity to effectively screen and manage any tumour and treatment‐associated complications. These requirements have made it difficult for many low and middle‐income countries (LMIC) centres to offer curative treatment. This article provides management recommendations for children with standard‐risk medulloblastoma (localised tumours in children over the age of 3–5 years) according to the level of facilities available. Pediatr Blood Cancer 2015;62:553–564.

The burden of sickle cell disease in Cape Town.

BACKGROUNDnSouth Africa has a low incidence of sickle cell disease (SCD). However, its demographics are changing because of immigration from sub-Saharan African countries where SCD is prevalent.nnnOBJECTIVESnWe aimed to determine the frequency of SCD presenting to the Haematology/Oncology Service at Red Cross War Memorial Childrens Hospital in Cape Town and to measure the associated disease burden.nnnMETHODSnThis was a retrospective cross-sectional study of patients first attending the Haematology Service between January 2001 and June 2010.nnnRESULTSnA total of 58 SCD patients were identified, with an annual frequency that increased over the study period by 300 - 400%. Up to 93.1% (n=54) were originally from other African countries, mainly the Democratic Republic of Congo (62.1%, n=36). One patient had sickle D-Punjab genotype, and all the other patients had the homozygous sickle cell anaemia genotype (Hb SS). Their haematological parameters demonstrated a normocytic anaemia with high white cell counts. The mean number of clinic visits per patient per year was 22.2 (range 0 - 64), and the mean number of hospital admissions per patient per year was 1.2 (range 0 - 5). All the patients were on antibiotic prophylaxis. The majority had at least one blood transfusion (65.5%, n=38), and a significant proportion required intravenous analgesia on admission (29.3%, n=17) and hydroxyurea treatment (36.2%, n=21).nnnCONCLUSIONSnOver the past 10 years the frequency of SCD has increased considerably, imposing a significant burden and new challenges to the health services in Cape Town.

Kawasaki disease preceding haemophagocytic lymphohistiocytosis: Challenges for developing world practitioners

Kawasaki disease (KD) is a recognised precipitant of haemophagocytic lymphohistiocytosis (HLH). Although KD has been previously described in the developing world, there are no reported cases of KD preceding HLH. We report a case of a child with a persistent rash and unremitting fever consistent with the diagnosis of KD, who was found to have HLH, after intravenous gamma globulin failed to produce a clinical response. The diagnosis was made using the revised diagnostic criteria for HLH from the Histiocyte Society (1994). She fulfilled six of the eight clinical and laboratory criteria needed to make the diagnosis. Pediatr Blood Cancer 2010;54:1023–1025

Pulmonary Kaposi sarcoma in six children.

BackgroundPulmonary involvement in Kaposi sarcoma is rare in children and can be difficult to distinguish from other pathology.ObjectiveTo describe the radiological findings in paediatric pulmonary Kaposi sarcoma.Materials and methodsSequential chest radiographs of six children and CT scans of four of these children were evaluated retrospectively. Their ages ranged from 18xa0months to 10xa0years; four were male and two were female. All six children were HIV-positive. The observers were two radiologists.ResultsChest radiographs revealed air-space (100%) and reticular (83%) opacification in the mid- and lower lung zones; pleural effusions were present in 83% of the children. All the children showed progressive air-space opacification on follow-up radiography. CT demonstrated bilateral air-space opacification in a perihilar distribution in all the children; reticular opacification was seen in 75%. All the children had mediastinal and axillary lymphadenopathy; 75% had bilateral hilar lymphadenopathy.ConclusionIn both adults and children, chest radiography demonstrates perihilar and lower zone involvement. Pleural effusions are more common on radiographs in children. Air-space disease and lymphadenopathy are much more common on CT in children than adults.

Nephron-sparing surgery for bilateral Wilms' tumours: a single-centre experience with 23 cases.

INTRODUCTIONnThe challenge of management with bilateral Wilms tumours is the eradication of the neoplasm, while at the same time preserving renal function. Surgical management with a variety of nephron-sparing techniques, combined with chemotherapy and occasionally supplemented by transplantation has evolved over the last 30 years to achieve remarkable success. We document the experience of a single centre in a developing country.nnnMATERIAL AND METHODSnTwenty-three bilateral Wilms tumours were seen in our service between 1981 and 2007. Treatment was, in most cases, according to National Wilms Tumour Study Group protocols, with initial bilateral biopsy, neoadjuvant chemotherapy, and tumourectomy. Technique of nephrectomy included full mobilization of the tumour-involved kidney, topical cooling with slush ice, vascular exclusion, tumour resection and reconstruction of the remnant kidney.nnnRESULTSnTwelve patients are alive and free of disease one to 15 years after treatment, all with well-preserved renal function (lowest glomerular filtration rate was 65 ml/min per (1.73 m 2 ). None of the survivors have hypertension. Eleven have died (two of unrelated disease) including six of the seven with spread outside the kidney. All three with unfavourable histology are alive. Four of the five metachronous presentations are alive, as are eight of 12 patients with synchronous bilateral tumours who presented since 2000.nnnCONCLUSIONSnAppropriate chemotherapy and nephron-sparing surgery can achieve good results with preservation of adequate renal function in nearly all cases. Unfavourable histology did not have a reduced survival in our series. Metastatic spread outside the kidney had a poor prognosis.